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Spondyloarthritis Associated with Acne Conglobata, Hidradenitis Suppurativa and Dissecting Cellulitis of the Scalp: a Review with Illustrative Cases

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Spondyloarthritis Associated with Acne Conglobata, Hidradenitis Suppurativa and Dissecting Cellulitis of the Scalp: a Review with Illustrative Cases Curr Rheumatol Rep (2013) 15:346 DOI 10.1007/s11926-013-0346-y SERONEGATIVE ARTHRITIS (MA KHAN, SECTION EDITOR) Spondyloarthritis Associated with Acne Conglobata, Hidradenitis Suppurativa and Dissecting Cellulitis of the Scalp: A Review with Illustrative Cases Debbie T. Lim & Neena M. James & Sobia Hassan & Muhammad A. Khan # Springer Science+Business Media New York 2013 Abstract To review and highlight the association of acne and inadequately managed include: acne conglobata, conglobata, hidradenitis suppurativa, and dissecting cellulitis of hidradenitis suppurativa, and dissecting cellulitis of the scalp the scalp with inflammatory arthritic conditions, we report five [1–15, 16•, 17••, 18]. In this review, we first briefly describe illustrative patients with this association, and a review of the the dermatologic manifestations of these diseases, and provide literature. All our patients were African-American males, and five illustrative case reports of the patients encountered at our their skin disease present before the onset of arthritis. Both center to highlight the clinical spectrum of the associated asymmetric peripheral arthritis and axial disease can occur. The inflammatory arthritis. This is followed by the description of arthritis is usually insidious and lacks association with the clinical, laboratory and radiographic findings of the rheumatoid factor and HLA-B27. Imaging of peripheral joints associated inflammatory arthritis and its management. can reveal erosions, periosteal bone reaction and new bone formation. When the axial skeleton is involved, imaging can reveal sacroiliitis, syndesmophyte formation. NSAIDs, oral and Acne Conglobata intra-articular steroids, DMARDs and TNF alpha antagonists have all been used with success. Controlled trials with larger Acne conglobata is a highly inflammatory form of acne numbers of patients are needed to assess which treatment which usually presents as numerous comedones, nodules, options are the most effective for this group of patients. papules, pustules, interconnecting abscesses and draining sinus tracts with associated scarring of the skin. Deep ulcers Keywords Ankylosing spondylitis . Sacroiliitis . may form beneath the nodules leading to keloid-type scars. Spondyloarthropathy . Seronegative arthritis . Treatment . Occasionally, acne conglobata may develop in the setting of TNF-inhibitors . HLA-B27 . Acne conglobata . Hidradenitis acne vulgaris that had been dormant for many years [19]. suppurativa . Dissecting cellulitis of the scalp . Inflammatory The lesions are usually found on the face, neck, chest, upper arthritic arms, buttocks and thighs. It is most common in teenage males but can occur in either sex and into adulthood [20]. This condition is different from acne fulminans, the most Introduction severe form of nodular acne, which is often classified with acne conglobata in the medical literature. Initially, the Dermatological diseases that show an association with disease resembles acne conglobata with numerous lesions seronegative inflammatory arthritis but are under-recognized on the back and chest, although the neck and face are invariably spared. The distinguishing morphologic feature This article is part of the Topical Collection on Seronegative Arthritis is the formation of hemorrhagic nodules and plaques which D. T. Lim : N. M. James : S. Hassan : M. A. Khan (*) later ulcerate [21]. The onset of acne fulminans is more Division of Rheumatology, Case Western Reserve University explosive, nodules and comedones are less common, School of Medicine, MetroHealth Medical Center, ulcerative and crusted lesions are unique, and systemic 2500 MetroHealth Drive, Cleveland, OH 44109-1998, USA symptoms such as fever, leukocytosis, polyarthralgia, e-mail: [email protected] myalgia, hepatosplenomegaly, and anemia are more 346, Page 2 of 8 Curr Rheumatol Rep (2013) 15:346 common [20]. It almost exclusively occurs in young men Caucasian male with acne conglobata and associated aged 13–16 years [21]. arthritis in both ankles [3]. In 1981, one of us (M.A.K.) reported at the Cleveland Society of Rheumatology meeting of an African-American Hidradenitis Suppurativa patient with HLA-B27 negative Reiter’s-like reactive arthritis in association with hidradenitis suppurativa and Hidradenitis suppurativa, also known as acne inversa, is a pyoderma gangrenosum. Initially seen in 1971, a 20-year chronic and recurrent inflammatory skin disease that follow-up of this patient was presented in 1992 [4] and we initially presents as tender subcutaneous nodules which now provide a 40-year follow-up (Case 1). coalesce into deep dermal abscesses [22]. The lesions occur Rosner et al. described a similar spondyloarthropathy most frequently in apocrine-gland bearing areas of the skin association with hidradenitis suppurativa and acne such as axillae, inguinal, perianal and perineal, mammary conglobata [5]. Ten adult patients were seen, and all but and inframammary, and buttock regions [22, 23]. Long one were African-Americans. Similar case reports showed thought to be a result of inflammation within the apocrine the same trend [6–10, 14]. Dissecting cellulitis of the scalp, gland itself, many now believe the inciting factor is a rare suppurativa disease characterized by painful scalp follicular plugging with resultant occlusion and rupture nodules and alopecia, has also been described either alone [24]. The subsequent influx of inflammatory cells leads to or in combination with acne or hidradenitis, with abscess formation and fistulous tracts. The disease typically spondyloarthropathy [12, 29]. occurs after puberty, with a peak incidence in the second or third decades, and is significantly more common in females [22]. Some authors have suggested a higher prevalence Illustrative Cases among African-Americans, but this has not been substan- tiated by studies examining racial predilection [24]. Case 1 This case had previously been reported in 1981 and 1992, and we now provide another follow-up. He is an African-American, now 66 years old, who has had Dissecting Cellulitis of the Scalp hidradenitis suppurativa since age 16, affecting the groin and axillary regions. He initially saw one of us (M.A.K.) in Dissecting cellulitis of the scalp, also called perifolliculitis 1971 when he had presented with pain in the left wrist and capitis abscendens et suffodiens, is a rare, chronic, relapsing, hand, and episodes of pain and inflammation of the knees, suppurativa disease characterized by painful fluctuant scalp ankles and feet. He had a past history of an episode of nodules and abscesses that heal with patchy areas of scarring painful pustular lesions on his left leg that were diagnosed and alopecia [16•]. The lesions usually begin on the occiput or by skin biopsy as pyoderma gangrenosum. Gastrointestinal vertex as a folliculitis and then expand into patches of evaluation including barium enema and sigmoidoscopy perifollicular pustules, nodules, abscesses and sinuses [25]. were negative. He responded to treatment with short courses This condition predominantly occurs in African-American of prednisone, erythromycin and surgical excision of his men aged 20–40 years of age but has been described rarely in hidradenitis lesions. other ethnic groups as well as among females [25–27]. The In June 1981, he developed increasing groin drainage etiology is unknown, but follicular occlusion, immune- accompanied by dactylitis of his right third finger and mediated chronic inflammation, and infection have all been an inflammatory arthritis involving his right third implicated in the pathogenesis [28]. metacarpophalangeal (MCP) and proximal interphalangeal Dissecting cellulitis of the scalp may occur alone, but (PIP) joints. Aspiration from the right third MCP joint revealed when seen in combination with acne conglobata and a white count of 16,000 with 89 % polymorphonuclear cells hidradenitis suppurativa, is referred to as the follicular with negative gram stain and culture. One month later, he occlusion triad [16•, 29]. developed right groin and trochanteric pain with marked difficulty walking for which he was hospitalized. Physical examination demonstrated worsening of his acne and Historical Aspect increased drainage of his groin abscesses. Joint pain and tenderness of the third MCP and PIP joint of the right hand In 1959, Burns and Coleville [1] reported a case of a 16- persisted, and he was noted to have pain with external rotation year-old Caucasian male with fever, severe acne conglobata of the hip. Erythrocyte sedimentation rate was 28. He lacked and arthralgia, with no objective joint findings. On follow- rheumatoid factor and HLA-B27, and his ANA was initially up, he later did develop erosive arthritis of the right knee negative. Bone scan showed increased uptake in the [2]. There was also a report in 1961 of a 15-year-old symptomatic finger joints with mildly increased uptake over Curr Rheumatol Rep (2013) 15:346 Page 3 of 8, 346 the right hip joint. Hand films showed early erosive change in 2 weeks of rifampin and clindamycin in Dermatology clinic right MCP joint. Gram stain and culture of fluid obtained from for acne as well as dissecting cellulitis of the scalp and right hip aspiration was negative. He was then started on sycoses barbae of the chin area. He was currently using prednisone 18 mg/day with prompt improvement in his joint transretinoin cream to the face and fluocinonide 0.05 % symptoms as well as his skin lesions. His prednisone dose was cream on the scalp. gradually tapered off over
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